Abstract
Hemolytic uremic syndrome (HUS) is rare in neonates. We report the case of atypical HUS (aHUS) revealed by neonatal seizures. This 18-day-old baby presented with repeated clonus of the left arm and eye deviation. Four days earlier, she had suffered from gastroenteritis (non-bloody diarrhea and vomiting without fever). Her work-up revealed hemolytic anemia (120 g/L), thrombocytopenia (78 g/L), and impaired renal function (serum creatinine=102 μmol/L) compatible with the diagnosis of HUS. Levels of C3 and C4 in the serum were normal. Shiga-toxin in the stools as well as the IgM and IgG against Escherichia coli O157 were negative. ADAMTS 13 deficiency, inborn error of the cobalamin pathway, deficiency in the H and I protein, and factor H antibodies were excluded and we concluded in aHUS. Genetic screening of the alternative complement pathway was normal. Cerebral magnetic resonance imaging performed after 24 h and 1 week showed restricted diffusion areas with periventricular white matter ischemic-hemorrhagic lesions. Extensive infectious work-up was negative. Upon admission the baby received antiepileptic drugs and 2 days later C5 monoclonal antibody (eculizumab) and two transfusions of packed erythrocytes because the hemoglobin v...Continue Reading
References
Jul 3, 2010·Clinical Journal of the American Society of Nephrology : CJASN·Marina NorisGiuseppe Remuzzi
Aug 31, 2011·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sibylle TschumiGiacomo D Simonetti
Jan 17, 2012·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·C AmourouxD Morin
Sep 8, 2012·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Nesrin BesbasFatih Ozaltin
Oct 3, 2012·Nature Reviews. Nephrology·Julien ZuberUNKNOWN French Study Group for aHUS/C3G
Oct 3, 2012·Pediatrics·Mario GiordanoLoreto Gesualdo
Jan 12, 2013·Clinical Journal of the American Society of Nephrology : CJASN·Véronique Fremeaux-BacchiChantal Loirat
Feb 23, 2013·Journal of the American Society of Nephrology : JASN·Elena BresinUNKNOWN European Working Party on Complement Genetics in Renal Diseases
Apr 2, 2013·Nature Genetics·Mathieu LemaireRichard P Lifton
Jun 7, 2013·The New England Journal of Medicine·C M LegendreC Loirat
Aug 7, 2013·Nature Reviews. Nephrology·Fadi Fakhouri, Véronique Frémeaux-Bacchi
May 13, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Sally JohnsonC Mark Taylor
May 21, 2014·Pediatrics·Martin ChristmannWilfried Schneider
Aug 26, 2014·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Katell MichauxAnne-Laure Sellier-Leclerc
Mar 25, 2015·Clinical Chemistry and Laboratory Medicine : CCLM·Massimo Franchini
Apr 10, 2015·Clinical Journal of the American Society of Nephrology : CJASN·Caterina MeleMarina Noris
Apr 11, 2015·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Chantal LoiratUNKNOWN HUS International
May 29, 2015·Thrombosis and Haemostasis·T MiyataY Fujimura
Citations
Apr 2, 2019·Journal of Pediatric Hematology/oncology·Maëlle de Ville de GoyetNathalie Godefroid
Jul 18, 2017·Blood Purification·Enrico VidalClaudio Ronco
Mar 9, 2021·Frontiers in Pediatrics·Dorottya KelenKhalid Ismaili